๐ ็ธฝ็ฎ้ ๏ฝ ๐ ่ฑๆๅๆ๏ผๆฌ็ฏ๏ผ ๏ฝ ๐ ๅฎๆด็ฟป่ญฏ ๏ฝ โญ ็ฒพ่ฏ็ญ่จ
Kaposiform lymphangiomatosis
Kaposiform lymphangiomatosis
Kaposiform lymphangiomatosis is a rare recently delineated clinicopathological entity.1 Most patients are children with rare cases presenting in adults. There is a slight male predominance. The majority of cases present with involvement of the thorax (lungs and mediastinum) followed by bone, spleen and intraabdominal organs. Patients present with respiratory symptoms, pleural and pericardial effusions, anemia and bleeding due to consumptive coagulopathy as a result of thrombocytopenia. Involvement of the skin and subcutaneous tissues, is very rare. Mortality is high, up to 51% at 5 years mainly due to involvement of thoracic organs. Histologically, a proliferation of dilated lymphatic channels lined by flat endothelial cells and associated with aggregates of parallel oriented spindle-shaped endothelial cells with frequent intracytoplasmic hemosiderin.
Multifocal lymphangiomatosis with thrombocytopenia (cutaneovisceral angiomatosis with thrombocytopenia)
Clinical features
This condition is also known as cutaneovisceral angiomatosis with thrombocytopenia or infantile hemorrhagic angiodysplasia, and is characterized by multiple redโbrown to blue discrete papules, macules, plaques and nodules ranging in size from millimeters to several centimeters, predominantly affecting the trunk and extremities.1โ4 Many lesions are congenital but new ones continue to develop throughout childhood. Other sites involved include the gastrointestinal tract, lung, bone, liver, spleen, muscle
1866 Connective tissue tumors
and synovium. Thrombocytopenia is an associated phenomenon. Death from gastrointestinal bleeding and sepsis may occur. Presentation without cutaneous involvement is exceptional.5 A case misdiagnosed as immune thrombocytopenia due to the delayed appearance of cutaneous lesions has been described.6
Histologic features Microscopically, irregular dilated vascular channels involve the reticular dermis and subcutis and are lined by bland endothelial cells with hobnail morphology and focal intraluminal papillary projections.
Differential diagnosis Benign lymphangioendothelioma, Dabska tumor and hobnail hemangioma can show similar histologic features to multifocal lymphangiomatosis with thrombocytopenia. However, the former are usually solitary lesions lacking visceral involvement or thrombocytopenia.